The impact of hospitalization on mortality in patients with connective tissue disease-associated interstitial lung disease: a medical records review study

Abstract Background Interstitial lung disease (ILD) remains one of the most important causes of morbidity and mortality in patients with Connective Tissue Diseases (CTD). This study evaluated the impact of hospitalization on mortality in an ethnically and racially diverse cohort of CTD-ILD patients. Methods We conducted a medical records review study at Montefiore Medical Center, Bronx, NY. We included 96 patients and collected data on demographic characteristics, reasons for hospitalization, length of stay, immunosuppressant therapy use, and mortality. We stratified our patients into two cohorts: hospitalized and nonhospitalized. The hospitalized cohort was further subdivided into cardiopulmonary and non-cardiopulmonary admissions. Two-sample tests or Wilcoxon's rank sum tests for continuous variables and Chi-square or Fisher's exact tests for categorical variables were used for analyses as deemed appropriate. Results We identified 213 patients with CTD-ILD. Out of them, 96 patients met the study's inclusion criteria. The majority of patients were females (79%), and self-identified as Hispanic (54%) and Black (40%). The most common CTDs were rheumatoid arthritis (RA) (29%), inflammatory myositis (22%), and systemic sclerosis (15%). The majority (76%) of patients required at least one hospitalization. In the non-hospitalized group, no deaths were observed, however we noted significant increase of mortality risk in hospitalized group (p = 0.02). We also observed that prolonged hospital stay (> 7 days) as well as older age and male sex were associated with increased mortality. Conclusion Prolonged (> 7 days) hospital stay and hospitalization for cardiopulmonary causes, as well as older age and male sex were associated with an increased mortality risk in our cohort of CTD-ILD patients.

Rendimiento diagnóstico y morbimortalidad de las biopsias pulmonares quirúrgicas en enfermedad pulmonar intersticial difusa

Introducción: Se denomina Enfermedad Pulmonar Intersticial Difusa (EPID) a un conjunto heterogéneo de patologías caracterizadas por inflamación y fibrosis pulmonar. El diagnóstico basado en patrones clínicos o radiológicos puede, ocasionalmente, ser insuficiente para iniciar un tratamiento. La biopsia pulmonar quirúrgica es una alternativa cuando se requiere aumentar la precisión diagnóstica luego de discusión multidisciplinaria. Objetivo: Describir el rendimiento diagnóstico, morbilidad y mortalidad de las biopsias quirúrgicas pulmonares en un hospital público chileno. Pacientes y Método: Cohorte retrospectiva de todos los pacientes a quienes se realizó biopsia quirúrgica por diagnóstico de EPID entre los años 2010 y 2020, indicada por un comité multidisciplinario. Se excluyen procedimientos similares o biopsias con diagnóstico de EPID como hallazgo incidental. Resultados: 38 pacientes intervenidos, mediana de edad de 63 años, 47% femenino. Solo 1 (2,6%) paciente operado de urgencia, y 34 (89,5%) por videotoracoscopía. 5 (13,1%) pacientes presentaron morbilidad, en 4 de ellos fuga aérea, ninguno requiriendo intervención adicional. No hubo rehospitalización, reoperación ni mortalidad a 90 días. En el 95% de los casos se alcanzó un diagnóstico preciso de la EPID tras discusión multidisciplinaria. Discusión: Se observa un alto rendimiento diagnóstico y una baja morbimortalidad en los pacientes estudiados. La baja frecuencia de procedimientos de urgencia y la adecuada indicación en comité multidisciplinario puede haber contribuido a la baja morbilidad. Conclusión: La biopsia pulmonar quirúrgica en un hospital general tiene un alto rendimiento diagnóstico cuando se discute en comité multidisciplinario para precisar el diagnostico en EPID, con una baja morbimortalidad si se seleccionan adecuadamente los pacientes.

Background: Interstitial Lung Disease (ILD) is a heterogeneous group of diseases characterized by inflammation and fibrosis of the lung. Diagnosis based exclusively on clinical or radiologic patterns may be inaccurate, and if a reliable diagnosis cannot be made, surgical lung biopsy can be strongly considered to increase the diagnostic yield after multidisciplinary committee. Objective: To review the diagnostic results, morbidity, and mortality of surgical biopsies in a chilean public health institution. Patients and Method: Retrospective cohort of patients operated for diagnostic purposes for ILD between 2010 - 2020. Surgical biopsies done for other diagnoses were excluded. Results: 38 patients were included, with a median age of 63 years, 47% were female. Only 1 patient (2.6%) underwent emergency surgery and 89.5% underwent minimally invasive surgery techniques. 5 patients had some morbidity (13.1%), 4 of them being air leak. All complications were successfully managed conservatively. We had no readmission, reoperations, or 90-day mortality in this cohort. In 95% of the cases an accurate diagnosis of ILD was reached after multidisciplinary discussion. Discussion: In our experience surgical lung biopsy has a high diagnostic yield and a low morbidity and mortality. A low number of emergency procedures and accurate surgical indication by an expert committee could explain the low morbidity. Conclusion: Surgical lung biopsy in a general hospital reach a high diagnostic performance when discussed in a multidisciplinary committee to specify the diagnosis in ILD, with low morbidity and mortality if patients are properly selected.

Risk factors for clinically significant diffuse parenchymal lung abnormalities persisting after severe COVID-19 pneumonia

Background & objectives: The risk factors for clinically significant diffuse parenchymal lung abnormalities (CS-DPLA) persisting after severe coronavirus disease 2019 (COVID-19) pneumonia remain unclear. The present study was conducted to assess whether COVID-19 severity and other parameters are associated with CS-DPLA. Methods: The study participants included patients who recovered after acute severe COVID-19 and presented with CS-DPLA at two or six month follow up and control group (without CS-DPLA). Adults volunteers without any acute illness, chronic respiratory illness and without a history of severe COVID-19 were included as healthy controls for the biomarker study. The CS-DPLA was identified as a multidimensional entity involving clinical, radiological and physiological pulmonary abnormalities. The primary exposure was the neutrophil-lymphocyte ratio (NLR). Recorded confounders included age, sex, peak lactate dehydrogenase (LDH), advanced respiratory support (ARS), length of hospital stay (LOS) and others; associations were analyzed using logistic regression. The baseline serum levels of surfactant protein D, cancer antigen 15-3 and transforming growth factor-? (TGF-?) were also compared among cases, controls and healthy volunteers. Results: We identified 91/160 (56.9%) and 42/144 (29.2%) participants with CS-DPLA at two and six months, respectively. Univariate analyses revealed associations of NLR, peak LDH, ARS and LOS with CS-DPLA at two months and of NLR and LOS at six months. The NLR was not independently associated with CS-DPLA at either visit. Only LOS independently predicted CS-DPLA at two months [adjusted odds ratios (aOR) (95% confidence interval [CI]), 1.16 (1.07-1.25); P<0.001] and six months [aOR (95% CI) and 1.07 (1.01-1.12); P=0.01]. Participants with CS-DPLA at six months had higher baseline serum TGF-? levels than healthy volunteers. Interpretation and conclusions: Longer hospital stay was observed to be the only independent predictor of CS-DPLA six months after severe COVID-19. Serum TGF-? should be evaluated further as a biomarker.

Caracterización de la enfermedad pulmonar intersticial en los pacientes con esclerosis sistémica

Introducción: La esclerosis sistémica es una enfermedad autoinmune crónica, caracterizada por vasculopatía, activación del sistema inmune y aumento de depósitos de matriz extracelular. En los últimos años, el compromiso pulmonar ha cobrado gran importancia, ha pasado a ser la primera causa de muerte en estos pacientes. La afección pulmonar puede ocurrir como hipertensión o enfermedad pulmonar intersticial. La meta del tratamiento es detener el deterioro de la función pulmonar. Objetivo: Caracterizar las manifestaciones clínicas, imagenológicas y la función respiratoria en pacientes con esclerosis sistémica y enfermedad pulmonar intersticial. Métodos: Se realizó un estudio observacional, descriptivo de corte transversal en el período comprendido entre diciembre de 2018 y diciembre de 2019. En el Servicio de Reumatología para caracterizar la enfermedad pulmonar intersticial en pacientes con esclerosis sistémica. El universo estuvo constituido por 168 pacientes, diagnosticados con esa enfermedad y la muestra se conformó por 55 pacientes que cumplieron los criterios de inclusión establecidos. Resultados: La enfermedad pulmonar intersticial fue más frecuente en pacientes mayores de 40 años, del sexo femenino, piel mestiza, predominó la forma clínica difusa, el síntoma más frecuente fue la disnea de esfuerzo, la mayoría tuvo ANA positivo y el patrón tomográfico en panal de abejas. La capacidad vital forzada estaba disminuida con mayor frecuencia, se asoció a un comportamiento autoinmune positivo para anti-ScL-70. Conclusiones: Se caracterizó las manifestaciones clínicas y radiográficas de la enfermedad pulmonar intersticial fueron comprobadas por la utilidad de la tomografía computarizada y la espirometría para identificar la presencia de fibrosis pulmonar.

Introduction: Systemic sclerosis is a chronic autoimmune disease, characterized by vasculopathy, activation of the immune system and increased extracellular matrix deposits. In recent years, lung involvement has gained great importance, it has become the first cause of death in these patients. Lung involvement can occur as hypertension or interstitial lung disease. The goal of treatment is to stop the decline in lung function. Objective: To characterize the clinical and imaging manifestations and respiratory function in patients with systemic sclerosis and interstitial lung disease. Methods: An observational, descriptive, cross-sectional study was carried out from December 2018 to December 2019 in the rheumatology service to characterize interstitial lung disease in patients with systemic sclerosis. The universe consisted of 168 patients diagnosed with this disease and the sample was made up of 55 patients who met the established inclusion criteria. Results: Interstitial lung disease was more frequent in patients older than 40 years, female, mixed-race skin color, the diffuse clinical form predominated, the most frequent symptom was exertional dyspnea, the majority had positive ANA and the pattern honeycomb tomography. Forced vital capacity was more frequently decreased, associated with positive autoimmune behavior for Anti-ScL-70. Conclusions: The radiographic and clinical manifestations of PID were verified by the usefulness of computed tomography and spirometry to identify the presence of pulmonary fibrosis.

Research progress on the predictive role of serum Krebs von den Lungen-6 in pulmonary diseases / 中国职业医学

Early diagnosis of pulmonary diseases is of great significance for their prevention and treatment. Serum Krebs von den Lungen-6 (KL-6) assay can reflect the damage degree of alveolar epithelium and stromal tissue, and is simple, non-invasive and low-cost. Pervious study showed that the serum KL-6 level was higher in patients with various interstitial lung diseases (e.g. idiopathic pulmonary fibrosis and connective tissue disease, primary Sjögren's syndrome, rheumatoid arthritis, idiopathic inflammatory myopathy and systemic sclerosis combined with interstitial lung disease), non-small cell lung cancer, various pneumonias and chronic obstructive pulmonary disease compared to healthy controls. Therefore, serum KL-6 has good sensitivity and specificity for the early diagnosis of these diseases. Occupational pneumoconiosis is an interstitial lung disease with a well-established etiology. Pervious study has shown that serum KL-6 level was higher in patients with occupational silicosis, occupational asbestosis, and dust-exposed workers compared to healthy controls. However, due to the limited sample size and the inconsistent findings on different studies, further research is needed to study the role of serum KL-6 in the early diagnosis of pneumoconiosis. Future studies should increase the sample size, improve the detection methods for serum KL-6, explore its feasibility as an early diagnostic biomarker for occupational pulmonary diseases, and investigate the efficacy andvalue of its combined application with other biomarkers in the early diagnosis of various pulmonary diseases, including occupational lung diseases, to fully exploit its predictive role in pulmonary diseases.

A concise review of diagnosis and evaluation of interstitial lung abnormalities / 中华劳动卫生职业病杂志

Interstitial lung abnormalities (ILAs) refer to the subtle or mild signs of ILAs pulmonary parenchyma on chest HRCT scans, which are not yet sufficient to diagnose a certain interstitial lung disease, may be potentially compatible an early stage of the diseases. The signs of ILAs usually includes ground-glass opacities, reticular abnormakicies, honeycombing, traction bronchiectasis or non-emphysematous cysts. This article reviews the research progreses in the definition and classification, risk factors, prognosis, comorbidities and management of ILAs in combination with domestic and foreign literatures.

Traditional Chinese Medicine Syndrome of Primary Sjögren's Syndrome Patients with Interstitial Lung Disease / 中国实验方剂学杂志

ObjectiveTo summarize the characteristics of traditional Chinese medicine （TCM） syndrome in primary Sjögren's syndrome （pSS） patients with interstitial lung disease （ILD） and to explore associated factors. MethodA survey was conducted and pSS patients who were treated in TCM department of rheumatism at China-Japan Friendship Hospital from December 2018 to April 2022 were included. Tongue manifestations and syndromes of patients were recorded. pSS patients with ILD were classified into the pSS-ILD group and those without the ILD were included in the pSS-non-ILD group. The tongue manifestations， syndromes， and laboratory indexes were compared between the two groups， and logistic regression was used to explore the factors associated with pSS-ILD. ResultA total of 200 pSS patients were included， with 186 （93.0%） females， median age of 57 years， and median disease course of 60 months， of which 44 （22%） had pSS-ILD. In terms of tongue manifestations， pSS-ILD patients generally had dark/purple/stasis tongue， fissured tongue， and tongue with little fluid， thick coating， yellow coating， and greasy coating. The proportion patients with yellow coating was higher in pSS-ILD group than in the pSS-non-ILD group （χ2=4.799，P<0.05）. In terms of syndrome， more than 40% of pSS-ILD patients had Qi deficiency， Yin deficiency， phlegm-dampness， Qi stagnation， and/or blood stasis syndrome. As for Yin deficiency， liver-kidney Yin deficiency syndrome ranked the first. For Qi deficiency， lung Qi deficiency syndrome was most commonly seen. The proportion of patients with lung Qi deficiency was higher in the pSS-ILD group than in the pSS-non-ILD group （χ2=18.667，P<0.01）. As to laboratory indexes， compared with the pSS-non-ILD group， pSS-ILD group had high proportion of anti-SSA-positive patients （P<0.05） and high levels of C-reactive protein （CRP） （P<0.01）， complement C3 （χ2=4.332，P<0.05）， and complement C4 （P<0.05）. Logistic regression analysis showed that pSS with ILD was positively associated with lung Qi deficiency ［odds ratio （OR）=6.079， 95% confidence interval （CI） 2.585-14.298， P<0.01）］ and yellow coating （OR=5.260， 95% CI 1.337-20.692， P<0.05） and negatively associated with low C4 （OR=0.199， 95% CI 0.070-0.564， P<0.01）. ConclusionAbout 22% of pSS patients had ILD， and patients with pSS-ILD generally have Qi deficiency， Yin deficiency， phlegm-dampness， Qi stagnation， and/or blood stasis syndrome. Yellow coating， lung Qi deficiency and C4 level are factors associated with pSS combined with ILD.

Genome-wide DNA methylation and transcriptome expression profiles of peripheral blood mononuclear cells in patients with systemic sclerosis with interstitial lung disease / 中南大学学报(医学版)

OBJECTIVES@#This study aims to investigate the genome-wide DNA methylation and transcriptome expression profiles of peripheral blood mononuclear cells (PBMCs) in patients with systemic sclerosis (SSc) with interstitial lung disease (ILD), and to analyze the effects of DNA methylation on Wnt/β-catenin and chemokine signaling pathways.@*METHODS@#PBMCs were collected from 19 patients with SSc (SSc group) and 18 healthy persons (control group). Among SSc patients, there were 10 patients with ILD (SSc with ILD subgroup) and 9 patients without ILD (SSc without ILD subgroup). The genome-wide DNA methylation and gene expression level were analyzed by using Illumina 450K methylation chip and Illumina HT-12 v4.0 gene expression profiling chip. The effect of DNA methylation on Wnt/β-catenin and chemokine signal pathways was investigated.@*RESULTS@#Genome-wide DNA methylation analysis identified 71 hypermethylated CpG sites and 98 hypomethylated CpG sites in the SSc with ILD subgroup compared with the SSc without ILD subgroup. Transcriptome analysis distinguished 164 upregulated genes and 191 downregulated genes in the SSc with ILD subgroup as compared with the SSc without ILD subgroup. In PBMCs of the SSc group, 35 genes in Wnt/β-catenin signaling pathway were hypomethylated, while frizzled-1 (FZD1), mitogen-activated protein kinase 9 (MAPK9), mothers against DPP homolog 2 (SMAD2), transcription factor 7-like 2 (TCF7L2), and wingless-type MMTV integration site family, member 5B (WNT5B) mRNA expressions were upregulated as compared with the control group (all P<0.05). Compared with the SSc without ILD subgroup, the mRNA expressions of dickkopf homolog 2 (DKK2), FZD1, MAPK9 were upregulated in the SSc with ILD subgroup, but the differences were not statistically significant (all P>0.05). In PBMCs of the SSc group, 38 genes in chemokine signaling pathway were hypomethylated, while β-arrestin 1 (ARRB1), C-X-C motif chemokine ligand 10 (CXCL10), C-X-C motif chemokine ligand 16 (CXCL16), FGR, and neutrophil cytosolic factor 1C (NCF1C) mRNA expressions were upregulated as compared with the control group (all P<0.05). Compared with the SSc without ILD subgroup, the mRNA expressions of ARRB1, CXCL10, CXCL16 were upregulated in the SSc with ILD subgroup, but the differences were not statistically significant (all P>0.05).@*CONCLUSIONS@#There are differences in DNA methylation and transcriptome profiles between SSc with ILD and SSc without ILD. The expression levels of multiple genes in Wnt/β- catenin and chemokine signaling pathways are upregulated, which might be associatea with the pathogenesis of SSc.

Baricitinib improves pulmonary fibrosis in mice with rheumatoid arthritis-associated interstitial lung disease by inhibiting the Jak2/ Stat3 signaling pathway

Abstract Objective The study explored improvements in pulmonary inflammation and fibrosis in a bovine type II collagen-induced rheumatoid arthritis-associated interstitial lung disease mouse model after treatment with baricitinib and the possible mechanism of action. Methods A rheumatoid arthritis-associated interstitial lung disease mouse model was established, siRNA Jak2 and lentiviral vectors were transfected with human embryonic lung fibroblast cells. And the levels of relevant proteins in mouse lung tissue and human embryonic lung fibroblasts were detected by Western blotting. Results The levels of JAK2, p-JAK2, p-STAT3, p-SMAD3, SMA, TGFβR2, FN and COL4 were increased in the lung tissues of model mice (P < 0.5) and decreased after baricitinib intervention (P < 0.05). The expression levels of p-STAT3, p-SMAD3, SMA, TGFβR2, FN and COL4 were reduced after siRNA downregulation of the JAK2 gene (P < 0.01) and increased after lentiviral overexpression of the JAK2 gene (P < 0.01). Conclusion Baricitinib alleviated fibrosis in the lung tissue of rheumatoid arthritis-associated interstitial lung disease mice, and the mechanism of action may involve the downregulation of Smad3 expression via inhibition of the Jak2/Stat3 signaling pathway, with consequent inhibition of the profibrotic effect of transforming growth factor-β1.

Crosstalk between the JAK2 and TGF-β1 signaling pathways in scleroderma-related interstitial lung disease targeted by baricitinib

Abstract Background and objective Systemic sclerosis (SSc) is an immune-mediated rheumatic disease characterized by fibrosis and vascular lesions. Interstitial lung disease is an early complication of SSc and the main cause of death from SSc. Although baricitinib shows good efficacy in a variety of connective tissue diseases, its role in systemic sclerosis-related interstitial lung disease (SSc-ILD) is unclear. The objective of our study was to explore the effect and mechanism of baricitinib in SSc-ILD. Methods We explored crosstalk between the JAK2 and TGF-β1 pathways. In vivo experiments, SSc-ILD mice model were constructed by subcutaneous injection of PBS or bleomycin (7.5 mg/kg) and intragastric administration of 0.5% CMC-Na or baricitinib (5 mg/kg) once every two days. We used ELISA, qRT-PCR, western blot and immunofluorescence staining to evaluate the degree of fibrosis. In vitro experiments, we used TGF-β1 and baricitinib to stimulate human fetal lung fibroblasts (HFLs) and assessed protein expression by western blot. Results The vivo experiments showed that baricitinib notably alleviated skin and lung fibrosis, decreased the concentration of pro-inflammatory factors and increased those of the anti-inflammatory factors. Baricitinib affected the expression of TGF-β1 and TβRI/II inhibitiing JAK2. In the vitro experiments, following the culture of HFLs with baricitinib or a STAT3 inhibitor for 48 h, the expression levels of TβRI/II decreased. Conversely, with successful inhibition of TGF-β receptors in HFLs, JAK2 protein expression decreased. Conclusions Baricitinib attenuated bleomycin-induced skin and lung fibrosis in SSc-ILD mice model by targeting JAK2 and regulating of the crosstalk between the JAK2 and TGF-β1 signaling pathways.

Anti-SSA/SSB-negative primary Sjögren's syndrome showing different clinical phenotypes: a retrospective study of 934 cases

Abstract Background Currently, only a few studies have described the general characteristics of patients with primary Sjögren's syndrome (pSS) who tested negatives for anti-SSA and anti-SSB antibodies. We aimed to further investigate the clinical characteristics of these patients in a large sample. Methods Data from patients with pSS who were treated at a tertiary hospital in China between 2013 and 2022 were retrospectively analyzed. Clinical characteristics of the patients were compared between those with and without anti-SSA and anti-SSB antibody negativity. Factors associated with anti-SSA and anti-SSB negativity were identified by logistic regression analysis. Results Overall, 934 patients with pSS were included in this study, among whom 299 (32.0%) tested negative for anti-SSA and anti-SSB antibodies. Compared with patients testing positive for anti-SSA or anti-SSB antibodies, that testing negative for the two antibodies had a lower proportion of females (75.3% vs. 90.6%, p < 0.001) and thrombocytopenia (6.7% vs. 13.6%, p = 0.002), but a higher proportion of abnormal Schirmer I tests (96.0% vs. 89.1%, p = 0.001) and interstitial lung disease (ILD) (59.2% vs. 28.8%, p = 0.001). Anti-SSA and anti-SSB negativity was positively associated with male sex (odds ratio [OR] = 1.86, 95% confidence interval [CI]: 1.05, 3.31), abnormal Schirmer I tests (OR = 2.85, 95% CI: 1.24, 6.53), and ILD (OR = 2.54, 95% CI: 1.67, 3.85). However, it was negatively related to thrombocytopenia (OR = 0.47, 95% CI: 0.24, 0.95). Conclusion Approximately one third of pSS patients had anti-SSA and anti-SSB negativity. pSS patients testing negative for anti-SSA and anti-SSB showed a higher risk of abnormal Schirmer I tests and ILD, but a lower risk of thrombocytopenia.

Treatment of Connective Tissue Diseases-associated Interstitial Lung Disease by the Origin-restoring and Bi （痹）-alleviating Method / 中医杂志

It is believed that the fundamental pathogenesis of the connective tissue diseases-associated interstitial lung disease （CTD-ILD） is kidney essence deficiency， with lung collateral obstruction throughout the disease， and environmental toxin pathogen is the important causative factors for the development of CTD-ILD. This article proposed to restore origin and alleviate bi （痹） for CTD-ILD， for which restoring origin means tonifying the lungs， spleen and kidneys to bank up the roots and consolidate the original qi， with modified Erxian Decoction （二仙汤） plus Liu Junzi Decoction （六君子汤）； alleviating bi means expelling wind and dredging collaterals， and eliminating the mass to restore the smoothness of the lung collaterals， with paired medicines of Chuanshanlong （Dioscorea nipponica）-Dilong （Kalanchoe pinnata）， Vinegar-processed Sanleng （Sparganium stoloniferum）-Vinegar-processed Ezhu （Curcuma zedoaria）， and stem-type medicines， and emphasized on removing the environmental toxin pathogens to facilitate the recovery of healthy qi.

Progress in epidermal growth factor receptor-tyrosine kinase inhibitors-associated interstitial lung disease / 中国胸心血管外科临床杂志

@#The tyrosine kinase activity of epidermal growth factor receptor (EGFR) plays a key role in tumor cell proliferation, invasion, migration, and drug resistance. Studies have shown that non-small cell lung cancer patients with somatic driver gene EGFR mutations are sensitive to and can benefit from EGFR-tyrosine kinase inhibitors (EGFR-TKIs). Nevertheless, EGFR-TKIs-related adverse events should not be ignored. Common adverse events such as diarrhea, acne-like rash and paronychia are usually manageable; although the incidence of interstitial lung disease is low, once it occurs, it is a serious threat to patients' life, and its pathogenesis is still unclear. There is very limited animal experimental and clinical research evidence on the potential mechanism of EGFR-TKIs-related interstitial lung disease in the available literature. Based on this, this article reviews the association between EGFR-TKIs and interstitial lung disease, at the same time, also discusses the research progress of EGFR-TKIs-related interstitial lung disease in combination with cytotoxic drugs or immunotherapeutic drugs and EGFR-TKIs, in order to provide a reference for the prevention and treatment of EGFR-TKIs-related interstitial lung disease in clinical practice in the future.

Clinical Profile of Connective Tissue Disease- related Interstitial Lung Diseases at a Tertiary Care Center in Western India

Connective tissue disorders (CTDs) are one of the common causes of interstitial lung diseases (ILDs). This prospective observational study included around 51 patients of CTD-ILDs, and their demographic, clinical, radiological, and laboratory profiles were studied. The most common type of CTD-ILD in our study is rheumatoid arthritis-related ILD. On high-resolution computed tomography (HRCT) thorax, nonspecific interstitial pneumonia (NSIP) was the most common pattern seen in 30 patients (59%), followed by usual interstitial pneumonia (UIP) seen in 20 patients (39%). Even though CTD-ILDs are similar to other idiopathic ILDs in clinical and radiological presentation, patients with CTDs have to be evaluated clinically and radiologically for early diagnosis. Early treatment initiation and pulmonary rehabilitation help in delaying the progression of disease. Among all ILDs, CTD-ILDs are associated with better prognosis and survival

Pulmonary Capillary Hemangiomatosis without Pulmonary Arterial Hypertension Mimicking Interstitial Lung Disease

Pulmonary capillary hemangiomatosis (PCH) is a rare disease usually associated with pulmonary hypertension which is classified in group I as per the World Health Organization (WHO) classification of pulmonary arterial hypertension (PAH). It is commonly presented as effort intolerance and hypoxia. Pulmonary capillary hemangiomatosis can present with ground-glass opacity on chest radiograph which can mimic interstitial lung disease. We need to consider PCH also in the differential diagnosis of interstitial shadows with these clinical presentations and should consider diagnostic evaluation for the same in case of unsatisfactory clinic-radiological response to treatment. We describe a case presented and treated as interstitial lung disease, which turned out to be PCH on lung biopsy. Interestingly, the case was not associated with any evidence of PAH.

Cyclophosphamide Pulse Therapy in the Treatment of Systemic Sclerosis Associated Interstitial Lung Disease: An Observational Study

Introduction: Scleroderma is a multisystem autoimmune connective tissue disease with approximately 90% of patients having lung involvement. It is the leading cause of morbidity and mortality in scleroderma. There is no effective treatment once there is lung involvement in the form of fibrosis. Study setting: Conducted in a tertiary care center between January 2017 and December 2019. Aim: To evaluate the efficacy of intravenous cyclophosphamide in patients with scleroderma-associated interstitial lung disease (ILD). Study population: Symptomatic patient with scleroderma with high-resolution computed tomography (HRCT)-proven non-specific interstitial pneumonia (NSIP)-pattern ILD. Methodology: Patients received 12 cycles of cyclophosphamide at a dose of 10 mg/kg every 4 weeks. Patients were followed up for 1 year after treatment completion. A six-minute walk test (6MWT) and spirometry were done at baseline and then every 6 months up to 2 years. Diffusing capacity of lung for carbon monoxide (DLCO) was done at baseline and then yearly for up to 2 years. Results: A total of 38 patients completed the study. The majority of patients had diffuse cutaneous type of systemic sclerosis. Throughout the study period, there was a gradual worsening of dyspnea as measured by the Modified Medical Research Council (mMRC) scale. Mean forced vital capacity (FVC) improved with 1 year of treatment, but later steadily decreased during follow-up. Similarly, DLCO also improved during 1-year treatment, but the improvement was not sustained during follow-up. There was a statistically significant improvement in 6MWD at the end of 6 months. This was followed by a gradual fall in 6MWD during follow-up. The only adverse event noted was persistent leukopenia in one patient. Conclusion: Intravenous pulse cyclophosphamide therapy in patients with scleroderma-associated ILD is associated with stabilization of pulmonary function during the treatment period, but not maintained during follow-up.

Effect on the Prevalence of Various Diffuse Parenchymal Lung Diseases due to Paradigm Change in the Guidelines

Background: Diffuse parenchymal lung diseases (DPLDs) have gone through various changes in nomenclature and classification since they were first described in 1868. Increasing knowledge about their etiopathogenesis has since led to several reclassifications and changes in the nomenclature. This has had a major impact on the prevalence of each interstitial lung disease (ILD) reported by the different registries worldwide. In this study, we attempted to describe the distribution of the different DPLDs in our population and reported changes in prevalence due to changing diagnostic criteria for the disease. Materials and methods: We analyzed retrospective data of 434 patients. For the initial 75 patients, ATS/ERS guidelines published in 2002 were followed in the diagnosis of the ILD (group I). In the later part of the study (359 patients), the diagnosis was based on the computed tomography (CT) patterns defined by ATS/ERS/JPS/ALAT statement on diagnosis of idiopathic pulmonary fibrosis (IPF) and updated 2013 ATS/ERS guidelines (group II). Results: Of the 75 patients in group I, IPF was the most common diagnosis (52%) made at that time, followed by sarcoidosis and connective tissue-related ILD (CTD-ILD) with 12% each. Group II had 359 patients, with IPF again being the most commonly diagnosed ILD with 21.3%. This was followed by CTD-ILD (18.6%), sarcoid (14.7%), and idiopathic nonspecific interstitial pneumonitis (iNSIP; 13.3%). The changing guidelines have an impact on reporting of different DPLD by our multidisciplinary teamover a period of time. Though IPF was the most commonest DPLD reported among both the groups, the diagnosis of IPF had fallen by more than half in the second group. It was paralleled by an increase in the diagnosis of iNSIP and chronic hypersensitivity pneumonitis. These reported changes in the prevalence of DPLDs may reflect the better-defined criteria in the latest guidelines and a better understanding of the fibrotic ILDs other than IPF by the multidisciplinary team. Conclusions: The frequency of diagnosis of the different DPLDs has changed, following the publication of several guidelines in the last decade. It has recognized newer entities with greater clarity, such as idiopathic NSIP and interstitial pneumonia with autoimmune features.

Manifestaciones pulmonares de la artritis reumatoide, una revisión en tiempos de pandemia por SARS-CoV-2 / no

La artritis reumatoide (AR) es una de las patologías crónicas de origen autoinmune más frecuentes. Su prevalencia varía del 0,5 al 1%, con un compromiso primario a nivel articular, generando gran discapacidad por las deformidades secundarias derivadas de un estado inflamatorio persistente. Considerando el alto impacto en la calidad de vida de quienes la padecen, sumado al alto costo de las intervenciones terapéuticas, se vuelve imperativo para el personal de salud sumar todos los esfuerzos para promover un diagnóstico temprano y reconocer las potenciales complicaciones con el fin de impactar positivamente en los desenlaces clínicos. Alrededor del 50% de los pacientes con AR pueden tener compromiso extra articular, siendo el pulmón uno de los órganos más afectados. En época de pandemia por el virus SARS-CoV-2 es necesario recordar los tipos de compromiso pulmonar en pacientes con AR y tener en cuenta la susceptibilidad de estos pacientes a cuadros infecciosos que pueden generar una gran morbimortalidad.

Rheumatoid arthritis (RA) is one of the most frequent chronic autoimmune pathologies. It's prevalence varies from 0.5 to 1%, with a primary involvement at the joint, generating disability due to deformities secondary to persistent inflammation. Considering the high impact on the quality of life of those who suffer it, added to the high cost of therapeutic interventions, it becomes imperative for health personnel to join forces to promote early diagnosis and recognize potential complications, in order to impact positively on clinical outcomes. Around 50% of patients with RA may have extra-articular involvement, the lung being one of the most affected organs. In times of SARS-CoV-2 pandemic, it's necessary to remember the types of lung involvement in patients with RA and take into account the susceptibility of these patients to infectious conditions that can generate great morbidity and mortality.

Caracterización de los hallazgos tomográficos en pacientes con la COVID 19 y sospecha de fibrosis pulmonar

Introducción: La evaluación tomográfica del tórax permite conocer las complicaciones en pacientes con COVID-19, es la fibrosis pulmonar una de las más temidas, por su repercusión en la funcionabilidad pulmonar. Objetivo: Caracterizar los hallazgos tomográficos en pacientes con COVID-19 y sospecha de fibrosis pulmonar. Métodos: Se realizó un estudio observacional descriptivo retrospectivo en el Hospital Cubano de Qatar, en el período comprendido entre abril de 2020 a abril de 2021, el universo de estuvo constituido por 129 pacientes con COVID-19, la muestra quedó conformada por 85 pacientes con sospecha radiográfica de fibrosis pulmonar. La información se obtuvo de las historias clínicas. Se consideraron los principios bioéticos. Resultados: Predominó el sexo masculino, el grupo de edad de 45-59 años. El patrón de vidrio deslustrado fue el más frecuente en el 60 % de los casos. Existieron signos tomográficos de fibrosis pulmonar en el 58,62 %, con diagnóstico en fase avanzada, extensión moderada, localización bilateral y difusa. La afectación multilobar fue la más frecuente en pacientes con fibrosis evidente y en los dudosos de fibrosis pulmonar. Conclusiones: Predominaron los pacientes del sexo masculino con edades entre 45-59 años. El patrón de vidrio deslustrado fue el más frecuente. En más de la mitad de los pacientes se evidenciaron signos de fibrosis pulmonar en la fase avanzada de la COVID-19, con extensión moderada.

Introduction: The tomographic evaluation of the thorax allows us to know the complications in patients with COVID-19, pulmonary fibrosis being one of the most feared, due to its impact on lung function. Objective: To characterize the tomographic findings in patients with COVID-19 and suspected pulmonary fibrosis. Methods: A retrospective descriptive observational study was carried out at the Cuban Hospital in Qatar, between April 2020 and April 2021, the universe consisted of 129 patients with COVID 19, the sample was made up of 85 patients with radiographic suspicion of Pulmonary Fibrosis. The information was obtained from the medical records. Bioethical principles were taken into account. Results: The male sex prevailed, the age group of 45-59 years. The ground glass pattern was the most frequent in 60% of the cases. There were tomographic signs of Pulmonary Fibrosis in 58.62%, with diagnosis in advanced phase, moderate extension, bilateral and diffuse location. Multilobar involvement was the most frequent in patients with evident fibrosis and in those with doubtful pulmonary fibrosis. Conclusions: Male patients aged between 45-59 years predominated. The ground glass pattern was the most frequent. In more than half of the patients, signs of pulmonary fibrosis were evidenced in the advanced phase of COVID -19, with moderate extension. Bilateral and diffuse localization predominated. Multilobar involvement was the most frequent in patients with evident fibrosis and in those with doubtful pulmonary fibrosis. The contribution of this research lies in the fact that with the characterization of the tomographic findings in patients with COVID 19, future complications are prevented.

A vexing case of non-healing ulcers with interstitial lung disease

Clinically amyopathic dermatomyositis is a subset of dermatomyositis that does not have any clinical evidence of muscle inflammation. Hence, it frequently poses a diagnostic challenge to the clinician. Here, we present a middle-aged farmer who presented only with multiple non-healing ulcers and was eventually found to be having early interstitial lung disease. He was finally diagnosed with anti-melanoma differentiation-associated gene 5 dermatomyositis and was started on aggressive immunosuppressants.